Notices: 39th Annual Meeting of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition Dresden, Germany, June 7-10, 2006: Abstracts: H-04
Serum autoantibodies define distinct subtypes of autoimmune hepatitis (AIH) and play a major role in diagnosis. A few patients, however, occasionally present with a cryptogenic liver disease responsive to immunosuppressive treatment in absence of serum autoantibodies making the diagnosis of AIH, difficult. We report a series of 12 children from 2 pediatric liver centres with a seronegative cryptogenic liver disease with features of AIH suggesting that AIH may be serologically silent in childhood.
Retrospective analysis of patients with cryptogenic liver disease with features of AIH, but without serum autoantibodies observed in 2 paediatric liver centres from 1990.
Patients were 8 girls and 4 boys; age at diagnosis ranged from 25 to 197 months. Family history for immune-mediated disorders was present in 4; 9 patients presented with acute hepatitis (7 icteric) and 3 with an asymptomatic hepatomegaly and/or abnormal liver enzymes. All patients had elevation of aminotransferase activity (2 to 72×N), 6 had high serum IgG related to age, 8 had a prothrombin activity <70% (median 41%). Serum autoantibodies (ANA, SMA, LKM LC1, ANCA, AMA, SLA) were negative in all at diagnosis. All known causes of acute and chronic liver damage were excluded. Liver biopsy showed interface hepatitis in all, multilobular/centrilobular necrosis in 6, portal fibrosis in 7 and cirrhosis in 2. Immune-mediated extra-hepatic disorders were present or developed during follow-up in 7 (bone marrow aplasia, autoimmune thrombocytopenia, type 1 diabetes mellitus, celiac disease, ulcerative colitis, autoimmune thyroiditis and autoimmune haemolytic anaemia). Initial treatment (prednisone w/w azathioprine or cyclosporine) was associated, within 3 months, with a complete remission in 9 and partial remission in 3 patients. Significant, but reversible side effects occurred in 6. Currently, all patients are alive and in complete remission with a median follow-up of 43 months: 11 on immunosuppressive treatment and 1 free of treatment since 87 months. Following the post-treatment scoring system of the International AIH Group, 8 patients scored as probable and 4 as definite AIH.
In children AIH may present without serum autoantibodies, commonly thought necessary for diagnosis, acute hepatitis is the most frequent type of onset, urgent liver biopsy looking for features compatible with AIH is needed to decide on an appropriate immunosuppressive treatment.